Sickle cell anemia is a blood disorder characterized by anemia arising from low levels of hemoglobin and hematocrit (packed red cell volume).
This form of anemia is a hereditary defect resulting from a genetic mutation, wherein a hemoglobin variant (sickle cell) is synthesized instead of the normal adult hemoglobin. In the generic sense, sickle cell disease applies to disorders characterized by human red blood cells, which contain an abnormal hemoglobin, that has been designated hemoglobin S.
When deprived of oxygen, sickle cell hemoglobin becomes insoluble, and forms hemoglobin crystals. These crystals form rigid rods which distort the normally, nearly spherical red blood cells to the sickle shaped forms. It has been found that sickle cells are more fragile than normal red blood cells, and the abnormal shapes of these cells cause them to be easily hemolyzed (broken) in circulating blood.
It has also been found that, in the circulating blood system, the sickled cells are destroyed by the phagocytes of the body's immune system. The rapid loss of the sickled cells leads to low hemoglobin and hematocrit and this precipitates a crisis in the sickle cell patient.
Hemoglobin is the oxygen-carrying protein in the red blood cells, and, in sickle cell anemia, the low hemoglobin, due to the loss of red blood cells, causes an inadequate supply of oxygen for bodily functions. Consequently, in an uncontrolled sickle cell condition, weakness and difficulty in breathing occur, and the sickled cells tend to clog narrow capillaries and cause blood clots which give rise to severe crippling pain in the parts of the body affected.
In the United States, blood transfusions and chemotherapy are the most widely used modes of sickle cell anemia control. In the case of transfusions, unfavorable side effects and risks render it unsafe for long term treatment. For example, in blood transfusion therapy, there are the risks of: transmission of hepatitis; acquired immune deficiency syndrome (AIDS); adverse transfusion reactions; ABO incompatibility; and bacterial contamination.
In the chemotherapy treatment for sickle cell anemia, many of the drugs expose the patient to the risk of toxicity and/or causes the sickled cell to be easily hemolyzed (broken) at the levels of effective usage, or results in other untoward side effects.
It is an object of the present invention to provide a process for effectively clinically treating sickle cell anemia patients without the risks attendant to the complicated process of blood transfusion.
Another object of the invention is to provide a composition for effectively clinically treating sickle cell anemia patients without the risks of toxicity associated with chemotherapy.
A yet further object of the invention is to provide a composition for effectively clinically treating sickle cell anemia patients without the risk of hemolysis or breaking the abnormal cells.
These and other objects of the invention will become more apparent from the invention hereinafter set forth.